Sellar atypical teratoid rhabdoid tumor in an adult: Clinical and pathological dilemmas

An atypical teratoid rhabdoid tumor (ATRT) is a pediatric embryonic tumor of the central nervous system and is uncommon in adults. We report a case of a 33-year-old female who presented with multiple dural lesions that were diagnosed as ATRT. She had a past history of endoscopic transnasal transsphenoidal and subsequent transcranial decompression of suprasellar lesion 6 months prior, with a presumptive diagnosis of atypical pituitary adenoma, which on retrospective evaluation was confirmed as sellar ATRT. Adult sellar ATRT, though rare, has now been proposed as a distinct clinicopathological and genetic variant that is predominantly seen in middle-aged women. We discuss the uniqueness of this rare aggressive tumor with reference to the age, location, and the challenges faced in the clinical and pathological diagnosis.